Glutaryl-CoA dehydrogenase (original) (raw)

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Glutaryl-CoA dehydrogenase (GCDH) is an enzyme encoded by the GCDH gene on chromosome 19. The protein belongs to the acyl-CoA dehydrogenase family (ACD). It catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and carbon dioxide in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. The enzyme exists in the mitochondrial matrix as a homotetramer of 45-kD subunits. Mutations in this gene result in the metabolic disorder glutaric aciduria type 1, which is also known as glutaric acidemia type I. Alternative splicing of this gene results in multiple transcript variants.

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dbo:abstract Glutaryl-CoA dehydrogenase (GCDH) is an enzyme encoded by the GCDH gene on chromosome 19. The protein belongs to the acyl-CoA dehydrogenase family (ACD). It catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and carbon dioxide in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. The enzyme exists in the mitochondrial matrix as a homotetramer of 45-kD subunits. Mutations in this gene result in the metabolic disorder glutaric aciduria type 1, which is also known as glutaric acidemia type I. Alternative splicing of this gene results in multiple transcript variants. (en) La glutaril-CoA deidrogenasi è un enzima appartenente alla classe delle ossidoreduttasi, che catalizza la seguente reazione: + accettore ⇄ + CO2 + accettore ridotto È una flavoproteina. (it) グルタリルCoAデヒドロゲナーゼ(glutaryl-Coenzyme A dehydrogenase)は、グルタリルCoAをクロトニルCoAに変換する酵素である。脂肪酸代謝、リシン分解、トリプトファン代謝に関与する酵素で補因子としてFADを用いる。 (ja) Глутарил-КоА-дегідрогеназа (англ. Glutaryl-CoA dehydrogenase) – білок, який кодується геном GCDH, розташованим у людей на короткому плечі 19-ї хромосоми. Довжина поліпептидного ланцюга білка становить 438 амінокислот, а молекулярна маса — 48 127. Послідовність амінокислот A: АланінC: ЦистеїнD: Аспарагінова кислотаE: Глутамінова кислотаF: ФенілаланінG: ГліцинH: ГістидинI: ІзолейцинK: ЛізинL: ЛейцинM: МетіонінN: АспарагінP: ПролінQ: ГлутамінR: АргінінS: СеринT: ТреонінV: ВалінW: Триптофан Y: Тирозин Цей білок за функцією належить до оксидоредуктаз. Задіяний у таких біологічних процесах, як ацетилювання, альтернативний сплайсинг. Білок має сайт для зв'язування з ФАД, флавопротеїном. Локалізований у мітохондрії. (uk)
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rdfs:comment Glutaryl-CoA dehydrogenase (GCDH) is an enzyme encoded by the GCDH gene on chromosome 19. The protein belongs to the acyl-CoA dehydrogenase family (ACD). It catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and carbon dioxide in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. The enzyme exists in the mitochondrial matrix as a homotetramer of 45-kD subunits. Mutations in this gene result in the metabolic disorder glutaric aciduria type 1, which is also known as glutaric acidemia type I. Alternative splicing of this gene results in multiple transcript variants. (en) La glutaril-CoA deidrogenasi è un enzima appartenente alla classe delle ossidoreduttasi, che catalizza la seguente reazione: + accettore ⇄ + CO2 + accettore ridotto È una flavoproteina. (it) グルタリルCoAデヒドロゲナーゼ(glutaryl-Coenzyme A dehydrogenase)は、グルタリルCoAをクロトニルCoAに変換する酵素である。脂肪酸代謝、リシン分解、トリプトファン代謝に関与する酵素で補因子としてFADを用いる。 (ja) Глутарил-КоА-дегідрогеназа (англ. Glutaryl-CoA dehydrogenase) – білок, який кодується геном GCDH, розташованим у людей на короткому плечі 19-ї хромосоми. Довжина поліпептидного ланцюга білка становить 438 амінокислот, а молекулярна маса — 48 127. Послідовність амінокислот A: АланінC: ЦистеїнD: Аспарагінова кислотаE: Глутамінова кислотаF: ФенілаланінG: ГліцинH: ГістидинI: ІзолейцинK: ЛізинL: ЛейцинM: МетіонінN: АспарагінP: ПролінQ: ГлутамінR: АргінінS: СеринT: ТреонінV: ВалінW: Триптофан Y: Тирозин (uk)
rdfs:label Glutaryl-CoA dehydrogenase (en) Glutaril-CoA deidrogenasi (it) グルタリルCoAデヒドロゲナーゼ (ja) Глутарил-КоА-дегідрогеназа (uk)
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